Carb Metabolism 5 Flashcards
GAGS have what type of charge, which attracts what?
negative charges due to sulfates; attachs cations
What are 6 proteoglycans?
- chondriten sulfate
- heparin sulfate
- dermatin sulfate
- keratin sulfate
- heparin
- hyaloronic acid
Where is the protein moeity of proteoglycans synthesized? Where are the sugar residues are added to what residues iwhre?
rER
aspargine and serine: golfi
What are finished proteoglycan molecues linked to?
hyaluronic acid
Where are proteoglycans degraded?
lysosomes
If tehre is a defective proteoglycan-degradign hydrolase, substrates will accumulate in the lysosomes, results in what types of diseases?
mucupolysaccharidosis (MPS)
What are three types of mucopolysaccharidoes?
Hunters: Dermatan sulfate
Heparan sulfate
Hurler Scheie: dermatan sulfate; hepara sulte
Sanfilippos : hep sul
For the glycosylation of proteins, the cell needs to synthesize what 3 things?
mannose, galactose, and fucose
What is the UGT reaction where the body can excrete poorly water-soluble molecules such as hemoglobin, steroid hormones and xebobiotics? (1 step). Use aspirin in this example
UDP-glucuronate + acetaminophen –> UGT–> UDP + x-glucuronate (less hydrophobic)
If the UDP-glucuronylation pathway of detox is overwhelm, acetaminophen is oxidized by what?
p450 cytochrome to NABQI
What is Crigler-Najjar Syndrome?
defect in UGT, so you can make is more water soluble; a buildup of unconjugated bilirubin leads to jaundice